Ataxin-3
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Subject Areas on Research
- Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117.
- Interaction of the polyglutamine protein ataxin-3 with Rad23 regulates toxicity in Drosophila models of Spinocerebellar Ataxia Type 3.
- Mutant huntingtin impairs PNKP and ATXN3, disrupting DNA repair and transcription.
- The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains.
- The ubiquitin-conjugating enzyme (E2) Ube2w ubiquitinates the N terminus of substrates.
- Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP.
- Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23.
- Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3.